| (Lager and Kroksmark, 2015)1
|
33 adolescents with DMD, 5 with Becker muscular dystrophy and 17 with spinal muscular atrophy; Age DMD group: 12–18 years’ old |
1 |
VAS, modified version BPI and questions of factors exacerbating and relieving pain |
Pain frequency, duration, location, intensity and discomfort, interference and factors exacerbating and relieving pain in the last three months |
Pain is a frequent problem in adolescents with dystrophinopathy and SMA and 69% report pain during the last three months. The pain prevalence does not differ significantly between the diagnostic groups or between ambulators and non-ambulators. The mean pain intensity is rated as mild and the worst pain as moderate, but with a wide range and some adolescents rate their pain as severe. The most frequently reported pain sites were the neck/back and legs. |
| (Pangalila et al., 2015)2
|
80 adults with DMD; Age: 20–44 years old |
1 |
Pain was assessed using the single-item ratings of pain of the Medical Outcomes Study 36-Item Short-Form Health Survey |
The presence of pain in the last four weeks, pain locations and the number of locations per individual, and how long pain had been present |
The frequent occurrence of pain, very often chronic, is not reflected in quality of life. The high frequency of complaints in the legs, arms, shoulders, and back suggests musculoskeletal pain. |
| (Sbrocchi et al., 2012)13
|
7 boys with DMD; Age: 8–14 years old |
Pre and post |
Boys report by tenderness on palpation over the site of the observed vertebral fractures |
Presence of back pain due to vertebral fracture |
Intravenous bisphosphonate therapy was associated with reduction in back pain and better stabilization to improve vertebral height ratios of previously fractured vertebral bodies. |
| 2 years of bisphosphonate therapy |
| (Bray et al., 2011)14
|
Parents of 34 boys with DMD and 90 boys with Charcot-Marie-Tooth (CMT); Age DMD group: 10–18 years old |
2 (Six months follow-up) |
Child Health Questionnaire parent form (CHQ-PF50) |
Bodily pain |
Boys with DMD and Charcot-Marie-Tooth type 1A are not different considering pain domain. DMD group presented amean score of 64.7 (bodily pain and discomfort domain) in both the first and second evaluation, which indicates moderate bodily pain. |
| (Takaso et al., 2010)15
|
20 boys with DMD; Age: 11–17 years old |
Before scoliosis correction surgery and after 6 weeks, 1 year, and 2 years |
Visual Analogue Scale (VAS) |
Back pain intensity |
All patients had back pain before surgery. Patients reported that difficulty sitting and back pain were alleviated after surgery. The mean VAS improved from 6.2 (before surgery) to 2.5, 1.8 and 1.6 after 6 weeks, 1 year and 2 years. |
| (Engel et al., 2009)6
|
14 youths with DMD, 6 with miotonic dystrophy, 2 with Becker dystrophy, 2 with limb-girdle dystrophy, 2 with congenital muscular dystrophy, 1 with facioscapulohumeral, and 15 were classified as “other NMD”; Age total: 8–20 years old |
1 |
11-point numerical rating scale and a modified Brief Pain Inventory (BPI) |
Pain frequency, intensity, location, quality, pain interference with function and quality of life, and interventions and their effectiveness. |
A total of 23 (55%) of the youths reported having chronic pain. Current pain intensity was 1.3 (range=0–6), mean pain intensity over the past week was 2.4 (range = 0–7), mean pain duration was 8.7 h (SD=12.8). Pain in the legs was most commonly reported and 83% reported using pain medications. This study indicates that chronic pain is a significant problem in youths with DMD. These data strongly support making comprehensive pain assessment and management an integral part of the standard of care for youths with NMD. |
| (Zebracki and Drotar, 2008)5
|
43 boys with DMD and 10 with BMD; Age DMD group: 8–18 years old. |
1 |
VAS, Likert scales, body outline markings, The Child Activity Limitations Interview, form indicating medical history and pain symptoms |
Pain symptoms, intensity, frequency, duration, location, emotional distress due to pain, and activity limitations due to pain |
Pain typically occurred at least once a week, with a mild-moderate range of intensity. Most children experienced pain for less than a few hours and little to moderate levels of emotional distress due to pain. Pain occurred most commonly in the lower back, spine, legs, and pelvic region, and was typically described as continuous pain. Children were able to manage their pain at least sometimes using techniques such as taking over-the-counter pain medications (e.g. acetaminophen, naproxen sodium), stretching, and shifting body positions. Pain typically occurred without warning at various times throughout the day, but did not disrupt sleep. Pain is a common occurrence in children with DMD or BMD, yet may be under-recognized. |
| (Guy-Coichard et al., 2008)7
|
128* youths with DMD and BMD (total 862 patients with NMD); Age DMD and BMD group**: Mean 32.8; Standard Deviation 12.5 |
1 |
Numeric scales for pain intensity and relief, BPI, Saint Antoine Pain Questionnaire, list of physical factors, body map |
Pain frequency, characteristics, topography, temporal features, factors influencing pain intensity and impact |
Pain is frequent in hereditary muscle disorders. From 128 patients, 85 reported pain in the last three months. The mean intensity of pain was mild and diffuse. Massage, physiotherapy and local applications of heat relieved pain. Worsening factors were vertical position, maintaining a fixed position and changes in position and physical exercise. DMD was one of the diseases with greatest impact of the pain on daily life. |
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